• 대한구순구개열학회지
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• 제5권1호
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• pp.21-25
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• 2002

Cleft lip and palate are most common congenital deformity to affect the orofacial region. Cleft lip and palate are caused by abnormal development of primary and secondary palate. It's causative mechanism is not completely understood, but genetic and environmental factors play an important role. Many epidemiologic surveys have been done extensively about incidence, racial influence, sex ratio, parent age, associated syndrome, and genetic factors. These researches are useful to dissolve many problems in prevention and treatment of cleft lip and palate. We performed epidemiologic survey of cleft lip and palate who visited the department of Oral & Maxillofacial surgery, Guro Hospital of Korea University from 1995 to 2001.

• Journal of Chest Surgery
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• 제29권4호
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• pp.466-471
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• 1996

기관식도루를 동반한 식도폐 쾌는 식도의 선천성 기 형중 흔한 형태로서 분만직후 이학적 검사상 간과 되는 것이 보통이다. 기관식도루를 동반한 식도폐쇄는 1696년에 Thomas Gibson이 처음 발표하였다. 본 원에서 4례의 선천성 하부기 관식도루를 동반한 식도폐쇄에서 우측 제 4늑간을 통하여 후흥막 접근법으 로 일차적 교정 술을 시 행하였다. 모든 환자에서 Haight 단단문합술을 시 행하였다. 수술후 2례의 환자에 서 문합부위의 심한 첩착소견을 보였고 1례의 환자에서 경한 협착소견을 보였다. 문합부위의 심한 협 착 이 있는i례의 환자에서 풍선식 식도확장술을시행하였다.

• Journal of Chest Surgery
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• 제20권2호
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• pp.300-308
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• 1987

Clinical and pathologic data were reviewed in 20 patients who had have surgery for isolated aortic valvular heart disease between April 1978 and April, 1987. Hospital mortality was 10%, with no additional late mortality during a mean follow-up period of 24.1 months. Prosthetic valve failure developed in 3 patients and two had reoperation. Niety four percent of the survivors who were in NYHA Funtional class III or IV before operation are now in class I or II. Ninety percent of all patients are still alive at a maximum follow up of 9 years. The clinical histories, gross and histologic examination of valves estabilished the causes for isolated aortic valve disease: 3 rheumatic, 2 congenital bicuspid, 2 hypertention, 2 aortitis and each one case of floppy valve, medial cystic necrosis of aorta, bacterial endocarditis. But etiology was unknown in 8 cases. Sixteen patietns had myxoid degeneration, defined as significant disruption of the valve fibrosa and its replacement by acid mucosaccharides and cystic changes. Myxoid degeneration was also the primary pathologic abnormality in the patients with 2 hypertention, 2 rheumatic, 1 aortitis, 1 bacterial endocarditis, 1 floppy valve, 1 congenital bicuspid. The patients with myxoid degeneration of uncertain origin were 8. Histologic finding of all of them revealed nonspecific patients with myxoid degeneration of uncertain orgin were 8. Histologic finding of all of them revealed nonspecific chronic valvulitis with myxoid degeneration. This finding may indicate that the etiology w uld be infectious.

• Journal of Chest Surgery
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• 제27권7호
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• pp.603-609
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• 1994

Esophageal atresia is a rare congenital anomaly and it usually associated with tracheo-esophgeal fistula and other congenital anomalies. The first report of esophageal atresia with tracheo-esophageal fistula was done by Thomas Gibson in 1696. In 1941, Haight performed the first successful primary anastomosis for esophageal atresia. These accomplishments opened the gateway for clinical studies that have resulted in reinforcements and improvement in the care of infants born with this anomaly. From January 1986 to April 1994, 14 cases of esophgeal atresia with tracheo-esophgeal fistula were diagnosed in Kyung Hee Uinv. Hospital. There were 9 male and 5 female infants. 12 infants were Gross classification type C and 2 infants were type A. The average body weight was 2.7$\pm$0.4kg and Waterson Category A contained 4 infants, B contained 3 infants and C contained 7 infants. Among these infants, 9 infants were underwent anastomosis procedures. We performed retropleural approach in 6 infants, transpleural approach in 2 infants and 1 infant was performed colon interposition through substernal space.By the method of anastomosis, end-to-side anastomosis was performed in 5 infants, end-to-end anastomosis in 3 infants and esophagocologastrostomy in 1 infant.The former 8 infants were Gross classification type C and the latter was type A. Among the type C infants, 6 infants were anastomosed with one layer interrupted suture and 2 infants with 2 layer interrupted suture. Post- operative death was in 1 infant and 8 infants were discharged with good result and have been in good condition.

• Clinical and Experimental Pediatrics
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• 제58권9호
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• pp.317-324
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• 2015

Kabuki syndrome (KS) is a rare syndrome characterized by multiple congenital anomalies and mental retardation. Other characteristics include a peculiar facial gestalt, short stature, skeletal and visceral abnormalities, cardiac anomalies, and immunological defects. Whole exome sequencing has uncovered the genetic basis of KS. Prior to 2013, there was no molecular genetic information about KS in Korean patients. More recently, direct Sanger sequencing and exome sequencing revealed KMT2D variants in 11 Korean patients and a KDM6A variant in one Korean patient. The high detection rate of KMT2D and KDM6A mutations (92.3%) is expected owing to the strict criteria used to establish a clinical diagnosis. Increased awareness and understanding of KS among clinicians is important for diagnosis and management of KS and for primary care of KS patients. Because mutation detection rates rely on the accuracy of the clinical diagnosis and the inclusion or exclusion of atypical cases, recognition of KS will facilitate the identification of novel mutations. A brief review of KS is provided, highlighting the clinical and genetic characteristics of patients with KS.

• Investigative Magnetic Resonance Imaging
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• 제20권2호
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• pp.114-119
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• 2016

We report a case of vegetation in a 4-year-old female with infective endocarditis, diagnosed by late gadolinium-enhanced (LGE) cardiovascular magnetic resonance (CMR) imaging. The patient had a history of primary closure for ventricular septal defect and presented with mild febrile sensation. No remarkable clinical symptoms or laboratory findings were noted; however, transthoracic echocardiography demonstrated a 14 mm highly mobile homogeneous mass in the right ventricle. On LGE CMR imaging, the mass showed marginal rim enhancement, which suggested the diagnosis of vegetation rather than thrombus. The extracellular volume fraction (${\geq}42%$) of the lesion was higher than that of normal myocardium. Based on the patient's clinical history of congenital heart disease and pathologic confirmation of the lesion, a diagnosis of infective endocarditis with vegetation was made.

• Journal of Chest Surgery
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• 제16권3호
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• pp.289-294
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• 1983

The report is concerned to our experience of 12 cases of open heart surgery under the extracorporeal circulation at the Department of Thoracic and Cardiovascular Surgery, Chosun University Hospital during the period between Nov, 1979 and April, 1983. 1. There were 4 cases of congenital anomaly and 8 cases of acquired heart disease. 2. There were 6 male and 6 female patients with a mean age of 20 years. [range 9 to 33 years]. 3. The cases induced 2 ventricular septal defect, 2 atrial septal defects and 8 acquired valvular heart diseases. 4. The surgical managements were 2 primary repair for atrial septal defect and 2 patch closure for ventricular septal defect, 1 triple valve replacement [AVR MVR TVR], 1 aortic valve replacement, 4 double valve replacement [AVR MVR] and 2 open mitral commissurotomy for pure mitral stenosis. 5. The average cardiopulmonary bypass time was 61.5 minutes for congenital heart disease and 201.4 minutes for acquired valvular heart disease and the average aortic cross clamping time was 36.75 minutes for the former and 165.6 minutes for the latter. 6. Postoperatively, there were 1 Alopecia, 1 Electric burn and 1 wound infection as complication. 7. Overall operative mortality was 8.3%. 7. All patients received valve replacement were recommended anticoagulation with persantin.

• Journal of Yeungnam Medical Science
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• 제11권1호
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• pp.35-41
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• 1994

저자들은 1988년 10월부터 1994년 2월까지 최근 5년 4개월간 영남대학교 의과대학 부속병원 일반외과에 입원하여 수술로서 확진된 선천성 회장 폐쇄증 환자 8명을 대상으로 임상분석하여 다음과 같은 결론을 얻었다. 1. 총 8명중 남아 4명, 여아 4명으로 남녀비가 동일하였으며, 입원시 연령은 생후 3일내가 6례로 75%를 차지하였다. 2. 선천성 회장폐쇄의 원인으로는 Type IIIa가 3례(37.5%)로 가장 많았으며, Type I 2례, Type II 2례, Type IIIb 1례 이었으며, Tape IV는 l례도 없었다. 3. 8명의 환아중 미숙아는 1명으로 저체중아였으며, 나머지 7명은 만삭아였다. 산모의 임신중 병력상 특별한 이상은 없었으며, 형제중 발생순위는 2째 아이에서 4례로 가장 많았다. 4. 주요 임상증상으로는 복부팽만과 구토로 7례에서 나타났고, 구토는 담즙성이었다. 5. 진단은 임상증상과 단순복부 촬영상 팽만된 장관 및 air-fluid level등을 보고 응급수술을 시행하였으며, 다른 질환과의 감별을 위해 2례에서는 대장조영술도 함께 시행 하였다. 6. 8명의 환아중 7명에서 장절제후 단단 문합술을 시행하였으며, 1명에서는 고위쇄항으로 횡행 결장조루술도 함께 시행 하였다. 7. 동반된 기형은 Type IIIa 1례에서 고위쇄항, 좌측신 무발육증, 우측 다낭신 및 후복막낭종등을 동반하였다. 8. 수술후 합병증은 8례중 3례(37.5%)에서 패혈증이 발생 하였으며, 술후 사망한 경우도 이들 패혈증을 동반한 3례 이었다. 나머지 5명은 현재까지 특별한 문제없이 건강하게 잘 자라고 있다.

• Journal of Chest Surgery
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• 제39권9호
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• pp.725-728
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• 2006

6개월 남아와 30개월 여아가 호흡곤란을 주소로 내원하였다. 이들은 모두 선천성 근질환을 진단 받았으며 반복적인 폐렴의 과거력이 있었고 흉부방사선촬영상 횡격막성 내장탈출을 관찰할 수 있었다. 근질환을 가진 환아에 있어서 일반적인 개흉술을 시행할 경우 환아의 술 후 합병증의 발생 위험이 높다고 판단되어 흉강경을 이용한 주름성형술을 시행하였다. 두 환아는 각각 수술 후 17일, 24일째 퇴원하였으며 현재 외래에서 경과 관찰 중이다. 본원에서는 선천성 근질환을 가진 환아를 대상으로 흉강경을 이용한 주름성형술 2예를 체험하였기에 보고하는 바이다.

• Journal of Chest Surgery
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• 제25권12호
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• pp.1383-1390
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• 1992

This is the result of the annual statistic analysis of thoracic and cardiovascular surgical cases in 1991, Korea. 14,715 cases of surgery[thoracic 8,995/cardiovascular 5,720] were done by 53 institutes replied. The order of frequency of cell type in primary lung cancer was squamous [62.3%] / adeno [23.9%] / small [6.4%] / adenosquamous [3.0%], and in mediastinal tumor, neurogenic[27.l%] / thymoma [27.1%] / teratoma[26.4%] / congenital cystic[12.0%]. Surgery for tuberculosis was decreased to 15.8% of overall infectious disease from the recent 6 year`s average 35.7%. In general thoracic surgery, the single most frequent operation was closed thoracostomy[4,047 cases] for pleural pathology. The ratio of congenital to acquired heart disease was 2:1, and acyanotic to cyanotic was 3:1. The order of frequency of congenital acyanotic heart disease was VSD [45.6%] / ASD [25.6%] / PDA [20.4%] / PS [2.9%], and that of cyanotic heart disease was TOF [42.6%] / PA [12.9%] / TGA [9.9%] / DORV [8.8%]. In 1,364 cases of valvular surgery, single mitral pathology was the most frequent candidate[729 cases, 53.4%]. In 243 cases of coronary surgery, bypassing graft materials were great saphenous vein[41.6%], internal mammary [39.5%], and artificial vessel[18.9%]. There were no specific differences in aortic surgery, assisted device implantation, and antiarrhythmic surgery as compared to previous study. This nation-wide inquiry will be continued and reported annually by KTCS Society.